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EARS, NOSE & THROAT (ENT)

Topic: Diseases of the Inner Ear


Lecturer: Dr. Caluag, Jim

Introduction:  Deafness can be assessed by tuning fork testing


If an infection involves the external ear, the common term that we use is  Headache, pain and fever may be absent.
otitis externa  A stiff neck accompanied by elevated spinal fluid pressure and an
If an infection involves the middle ear, it is known as otitis media increase in WBC count is not a good sign.
But if an infection involves the inner ear, it is called as labyrinthitis.
 A unilateral tympanogenic labyrinthitis produces more severe
 There is no such term as Otitis Interna
vestibular upset than a bilateral meningogenic labyrinthitis
LABYRINTHITIS This is because the patient is unable to adjust quickly enough to an
 Is an inflammatory process that involves both the auditory and acute labyrinthine failure
vestibular portions of the labyrinth giving rise to sensorineural hearing
loss and dizziness. Chronic Stage
 The inflammation may be localized to involve only the cochlea/auditory  ACUTE STAGE is followed by the CHRONIC STAGE or LATENT
labyrinth, which renders the patient to have hearing loss/deafness. LABYRINTHITIS characterized by FIBROBLASTIC PROLIFERATION within
 It may also localize to involve the vestibular labyrinth which will enable the inner ear fluid spaces.
the patient to complain of dizziness alone.  This chronic stage follows the initial stormy period and usually lasts
 The infection of the inner ear could come from other regions or other from 2-6 weeks.
structures.  By this time, the inner ear is already completely destroyed and deafness
 May spread from adjacent structures such as middle ear or mastoid is now complete.
(tympanogenic/otogenic labyrinthitis) or from meninges  Incapacitating vertigo has already subsided but a milder vestibular
(meningogenic labyrinthitis) upset and positional vertigo usually persist.
 Whether it is otogenic or meningogenic form, both disease entities  Audiologic, and vestibular studies revealed markedly reduced cochlear
involve the spread of infection first into the PERILYMPHATIC SPACES and vestibular function
causing extreme damage into the end organs of hearing and balance.  If bilateral meningogenic labyrinthitis has occurred, there will be
 Another way of the spread of the infection is through the bloodstream difficulty in ambulation, especially on soft surfaces
or HEMATOGENOUS spread and this is a special characteristic of viral
inflammations involving the inner ear. Healed Stage
Viral infection invades the labyrinth by the way of stria vascularis  This is followed by the third stage also known as the HEALED STAGE or
and the infection is called viral endolymphatic labyrinthitis giving COMPENSATED LABYRINTHITIS which is characterized by
rise to deafness resulting to damage to the end organs of hearing OSSIFICATION OF THE SO-CALLED LABYRINTHITIS OSSIFICANS.
 This period commences after 2-3 months or it takes years before
FORMS OF LABYRINTHITIS labyrinthitis ossificans will occur in the membranous labyrinth.
1.) Suppurative/Purulent Labyrinthitis  By this time, the patient has fully compensated the loss of auditory and
 Characterized by complete deafness or permanent hearing loss due to vestibular functions as per hearing and caloric testing
destruction of sensory areas of the labyrinth  Occasionally, positional vertigo may persist for some months during
 Consists of 3 Stages: this stage.
1. Acute Stage (Stormy Period)
 Characterized by the invasion of pus cells Management:
2. Chronic Stage (Latent Labyrinthitis)  Suppurative labyrinthitis must be actively treated during the ACUTE
 Characterized by fibroblastic proliferation within the STAGE with:
ear fluid spaces o Bedrest
3. Healed Stage (Compensated Labyrinthitis) o Intensive doses of Penicillin/Cephalosporins
 Characterized by ossification of the so called o Sulfa drugs and Quinolones
labyrinthitis ossificans o Rehabilitation in the Healed stage

Acute Stage:
 It starts with the ACUTE STAGE which is characterized by the invasion  If SNHL (sensorineural hearing loss) or vertigo is present, MODIFIED
of pus cells. RADICAL MASTOIDECTOMY must be done to eradicate Mastoid and
Middle Ear Diseases
 The acute stage is characterized by a stormy period lasting for 1-2
weeks wherein the vestibular symptoms peaks for the first few days
Complications:
then gradually subsides after a week.
 The most serious stage is the ACUTE STAGE because during this stage,
 When diffuse suppurative labyrinthitis occurs unilaterally, the patient
complications may occur by the spread of the infection to adjacent
has severe vertigo and the patient cannot stand or sit upright.
regions such as to the internal auditory canal along the nerves and the
 The patient must lie quietly on the side of the diseased ear which is
vessels or to the cochlear aquiduct.
completely deaf.
 Common complication is Septic Meningitis
 Slight head and body movements cause the patient to vomit.
o Extradural and cerebellar abscesses as well as sinus
 The surroundings seem to spin in the direction from the normal to the
thrombosis are possibilities
diseased side.
 The patient is very incapacitated to cooperate with tests that suggest a
diagnosis of the basic clinical picture.
 Caloric tests reveals impaired vestibular response
 Labyrinthitis should be differentiated from brain lesions
In labyrinthitis, labyrinthine nystagmus occurs for a few weeks and
then subsides whereas in a brain lesion nystagmus is noted for a
much longer duration

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EARS, NOSE & THROAT (ENT)
Topic: Diseases of the Inner Ear
Lecturer: Dr. Caluag, Jim

2.) Viral Labyrinthitis  Heavy metals (Gold, Lead, Mercury)


 It is usually seen in patients with measles, mumps and influenza.  Arsenic
 The virus gains entry to the endolymphatic spaces progressing via the  Aniline Dyes
3. Chemicals
stria vascularis and the condition is known as VIRAL ENDOLYMPHATIC  Alcohol
LABYRINTHITIS.  Tobacco
 Vestibular symptoms in VIRAL ENDOLYMPHATIC LABYRINTHITIS  Carbon Monoxide
usually is present during the onset but it subsides after about 2-3 days.  Bleomycin
4. Anti-cancer Drugs
 In viral labyrinthitis, deafness develops quickly and persists as a  Cisplatinum
permanent handicap.  Salicylates (Aspirin)
 Vestibular difficulty is not usually incapacitating for these viral disorders 5. Miscellaneous  Polybrene
so the patient usually does not consult to the doctor early in the course
Agents  Nitrogen mustard
 Quinine
of the disease necessitating a retrospective diagnosis after a time
relapse and the deafness of the patient has already become stable.
 There are certain drugs such as MANDELAMINE – induces tinnitus alone
 Since it is viral, treatment is supportive, symptomatic and non-specific.
 Streptomycin in large doses primarily destroys the Vestibular labyrinth
 It is a self-limiting condition and the patient will eventually recover.
sparing the Auditory Labyrinth although hearing will eventually
deteriorate
3.) Toxic (Serous) Labyrinthitis
It is important to monitor patients on streptomycin therapy for
 Also known as serous or irritative labyrinthitis
systemic diseases
 It is characterized by chemical changes in the inner ear fluid spaces but
there is no pus cell invasion.
Management:
 Serous labyrinthitis could occur after infection of bacterial toxins
 The most effective treatment for ototoxicity caused by drugs is to
involving the round and the oval window or after a meningeal infection.
withdraw from using the drug.
 It could also occur following an ear surgery
 The damage in ototoxicity is permanent.
 Spontaneous vertigo occurring in the presence of middle ear infection
 Hearing loss of the patient may be subjective but may not be supported
indicates serous labyrinthitis.
by clinical findings.
 If nystagmus is present – irritative type (the quick component is towards
 If upon examination, the patient complains of hearing loss bilaterally
the affected ear).
but upon inspection, both ears are normal, hearing loss may be
 There will be hearing impairment.
attributed to the use of such drugs mentioned above.
 Caloric Test – diminished vestibular response
 When vertigo or sensorineural hearing loss develops, erosion of the NEOPLASAMS OF THE INNER EAR
lateral semicircular canal or invasion of the round window membrane 1.) Malignant Lymphoma and Leukemia
is suspected.
 Only involves the temporal bone including the bone marrow of the
 Serous labyrinthitis causes less severe vertigo as compared to petrous apex as well as infiltrations involving the middle ear.
suppurative labyrinthitis in which the patient is severely incapacitated
by the rapid destruction of the end organs. 2.) Terminal Leukemia
 Management:  When leukemia becomes terminal, actual hemorrhage can occur
o Treatment involves administration of large doses of Penicillin involving the inner ear and complete deafness will result.
IV given in the meningitic dose of 2-3 million units per day.
o Symptomatic care which includes sedation and anti- 3.) Acoustic Neuromas
vertiginous drugs may help.
 Some of the most common tumors of the inner ear are tumors involving
o Surgery for more complicated cases
the CEREBELLO-PONTINE ANGLE such as ACOUSTIC NEUROMAS.
If granulation tissue is present in the M.E. or Mastoid, a
complete MASTOIDECTOMY must be done to alleviate the
Neoplasms
source of infection in the inner ear
 All patients complaining of dizziness, ringing of the ear, unilateral SNHL
and facial paralysis should have audiometric test for pure tone, a
OTOTOXICITY
careful caloric examination and radiologic examination of the
 Hearing loss (deafness and ringing of the ear) may result from
temporal bone including MRI and CT scan
detrimental effects of certain substances that involves the inner ear.
 The tumor can involve the vestibular nerve causing dysfunction
Agents Responsible for Causing Ototoxicity although true whirling vertigo is absent
 Aminoglycosides  There is generally a feeling of disequilibrium associated with other
 Chloramphenicol neurologic symptoms
 Ristocetin  Characteristic feature of an acoustic tumor – inability of the patient to
1. Antibiotics
 Macrolides understand speech (RECEPTIVE APHASIA)
 Pharmacetin  Once a tumor is suspected, a diagnostic test for auditory fatigue in
 Polymyxin B Sulfate
tumors of the internal auditory canal should be requested –
 Ethacrynic acid
CLIVOGRAM (Pantopaque Myelography) of the internal auditory canal
2. Diuretics  Furosemide
and posterior fossa
 Mannitol
Management:
 Removal of the tumor depends upon its biologic nature, location, size,
and symptom-causing disabilities
 Surgery varies from Middle Cranial fossae or Posterior Cranial fossae
approaches

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EARS, NOSE & THROAT (ENT)
Topic: Diseases of the Inner Ear
Lecturer: Dr. Caluag, Jim

TRAUMA SENSORINEURAL HERING LOSS


 Hearing loss may result from several forms of trauma such as noise  Quite a common complaint
exposure, explosive blasts and blows to the head and the ears.  It is a permanent and progressive disorder although there are cases of
 The fact that noise exposure can produce hearing loss has been known sensorineural hearing loss that can be reversible if the disease is
for a long time as Boilmaker’s deafness but lately a significance has identified and treated early.
been given to minor degrees of such hearing losses.  Examples include endolymphatic hydrops or Meniere’s Disease, or
 In general, an 85 decibel of noise exposure is injurious. other types of fluctuant hearing losses due to allergies, vascular
 Hearing loss caused by noise exposure results in extreme damage problems, serous or toxic labyrinthitis and ototoxicity.
involving the organ of Corti.  Hearing loss may also result from syphilis and hypothyroidism and
 The documented time of exposure to 80 decibel of noise is 54 minutes. proper treatment for the systemic disease will eventually result to
 If the noise is concentrated at 50 decibels, earphones should be used hearing improvement.
for less than 2 hours.  In the case of syphilis, the disease process may be localized to involve
 If a person is exposed to a 50 decibel noise for 6 hours, there is a only the inner ear and a trial period of high doses of Penicillin therapy
residual effect in the acoustic nerve and noise induced hearing loss can given intravenously will lead to improvement of the hearing function.
develop.  The main hope in dealing with sensorineural deafness lies in prevention.
 Animal research indicates that exposure to high intensity sounds for  When permanent sensorineural hearing loss is found, it is important to
long periods causes varying degrees of degeneration from slight provide follow up to the patient in order to assess the progression of
changes in the hair cells to complete degeneration and destruction of the hearing loss.
the Organ of Corti.  Audiology may be helpful in providing rehabilitation to the patient in
relation to the patient’s problems at work or in any social situation.
Factors Affecting Noise-Induced Deafness:  Hearing can be improved through amplification following hearing aid
 Overall level of noise education.
 Frequency composition of the noise  Hearing aid must be recommended by a professional in order for the
 Daily distribution of the noise patient to have satisfactory results.
 Total time of exposure to the noise  It is also important to teach patients lip reading along with hearing
amplification in order for them to function normally in the society.
Management:
 The most effective method of treatment is to avoid noise. TINNITUS
 The second most effective method of treatment is to reduce noise  Also known as ringing of the ears
exposure by wearing ear defenders (ear muffs).  A sound experience perceived by the patient originating with himself
Ear defenders - generally provides only up to 35 decibels of  Tinnitus heard at the center of the head indicates that the origination
protection. If the sound is intense enough, it can still be injurious in the two ears is of the same pitch and the loudest of the experience
practically arises from the neural apparatus of the central nervous
FRACTURES OF THE TEMPORAL BONE system.
 Bleeding caused by fracture cannot be controlled or treated by
applying pressure. 1.) Objective Type
 Manifests as a profusely bleeding ear  The tinnitus is heard not only by the patient but also by the examiner
 Any form of instrumentation must not be done unless sterile upon auscultation of the external auditory canal and the examiner will
instruments will be used because the injury might be further hear a sound which is usually described as a bruit type of tinnitus.
contaminated.  Causes of Objective Tinnitus:
 Fractures of the temporal bone may give rise to hearing loss and o Altered sound conduction
dizziness. This is associated with obstruction to sound conduction
seen in impacted cerumen, a middle ear fluid or a simple
1.) Longitudinal Fracture otosclerotic fixation of the stapedial footplate described as
 Considered to be less severe in causing deafness as compared to the a hollow sea-shell type of a sound due to masking of a well-
horizontal type functioning cochlear neural apparatus from the covering
effect of ambient sound
 May give rise to a concussion fracture in the inner ear resulting to
sensorineural hearing loss and usually there is a delayed onset of facial
o Vascular pathology
paralysis that would occur at about 24-48 hours
When a middle ear pathology exceedingly vascular as in a
 It is managed conservatively with steroid therapy and rehabilitation.
carotid body-like tumor, a pulsing sound of blood flow can
be heard as a troublesome tinnitus with exceedingly
2.) Horizontal or Transverse Fracture vascular and extensive otosclerotic foci
 The fracture line will extend to involve the internal acoustic meatus
going into the bony labyrinth. o Clonic contraction of middle ear muscles
 The bony labyrinth cannot heal by primary intention so secondary When the tensor-tympani and the stapedius develops
inflammation arises in the inner ear which gives rise to a profound clonic contraction, the result is a flutter or a machine gun-
sensorineural hearing loss and a dizziness together with an abrupt onset like sound in the ear. Nervous tension, stress, and fatigue
of facial paralysis. plays a part
 It is radically managed by doing mastoidectomy under general
anesthesia on an indication of facial nerve decompression. o Cervico-cranial vibrating phenomena
Vibratory phenomena perceived as an unpleasant sound
experience if the loudness is above the reception threshold
of the cochlear neural apparatus

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EARS, NOSE & THROAT (ENT)
Topic: Diseases of the Inner Ear
Lecturer: Dr. Caluag, Jim

Causes of Objective Tinnitus continued….. VERTIGO


o Patent Eustachian Tube  A patient with dizziness oftentimes comes to the clinic complaining of
Produces rushing sounds of air during respiration and lightheadedness, giddiness, imbalance or disequilibrium.
perceived as autophony by the patient, rapid weight loss  Vertigo is described as a sensation or hallucination of a twirling
and use of birth control pills are frequent factors motion.
 It is important to determine the cause of dizziness in order to establish
treatment and diagnosis.
2.) Subjective Type
 Tinnitus is heard only by the patient and is the most frequent type. Causes of Vertigo
 Causes of Subjective Tinnitus:  Refraction errors  Anemia
o Pathologic alterations in the cells of the end organ of Corti  Acoustic neuroma  Pregnancy
Pathologic disturbance in the hair cells on the basal turn of  Glaucoma  Postural hypotension
the cochlea results to high-pitched tinnitus described by  Brain lesion (tumors, infection  Chronic otitis media
the patient sounding like a bell ringing or trauma)  Hypertension
 Locomotor ataxia  Labyrinthitis (any form)
o Physical distortion of the cochlear sensory system  Tabes dorsalis  Toxic exposure
Creates a discordant cacophonic sound experience which is  Chronic alcoholism  BPPV
exceedingly distressing occurring in Meniere’s disease in  Multiple sclerosis  Vertebra-basilar insufficiency
which hearing impairment occurs first as lack of  Diabetes mellitus  Labyrinthine concussion
discrimination as a result of frequency distortion  Migraine  Meniere’s disease
 Hypoglycemia  Temporal bone fractures
o Endolymphatic hypertension  Seizures (grand mal)  Vestibular neuronitis
Results from functional vascular change in the terminals of  Cerebello-pontine angle lesions  Dehydration
the cochlear vascular system usually occurring in patients  Intracranial space occupying  Electrolyte imbalances
with fundamental endocrine imbalance lesions

o CNS disorders  Any disorder that causes unilateral reduction in vestibular function may
A neural apparatus central to the cochlea can be the origin cause vertigo
of tinnitus. Cerebello-pontine angle lesion affecting the 8th  The best way to establish whether vestibular activity is reduced,
nerve trunk directly generates a minor high pitched tinnitus vestibular function is measured by Caloric Test done by tilting the
not usually localized to the ear patient’s head 60 degrees backward and irrigating the ear with cold
water, a horizontal nystagmus of 1-3 minutes duration occurs with fast
Treatment for Tinnitus: component towards the opposite side
 Correction for a known cause is the most effective positive treatment  Most patients have very active response and the characteristic of
since therapy can be aimed specifically. nystagmus must be noted
 A bedside clock radio, loudly ticking alarm clock or a fan provides There are 3 characteristic of nystagmus exhibited by the patient:
ambient noise to mask the ringing at bedtime. 1. Vertical nystagmus – indicates brain lesion
 Tranquilizers and sedatives may be initially used but should be 2. Horizontal nystagmus – indicates that there is
discontinued as the patient becomes more adjusted to the symptoms. labyrinthine lesion
 The patient should be informed that there are no miracle drugs or 3. Rotary nystagmus – means that the vertigo is systemic
surgical procedures for tinnitus. in origin
 If there is an accompanying hearing loss, wearing hearing aids will
increase ambient sound to mask the tinnitus.  Vertigo due to CENTRAL DISORDERS – central vertigo may be due to:
o Multiple sclerosis
o Acoustic neuromas
o Seizures
o Basilar insufficiency
o Vascular accidents
 Several conditions affect the vertebral basilar arterial system and may
cause vertigo

Cervical Spondylitis
 May cause compression on the Basilar Artery resulting to vertigo
 Another condition is insufficient blood supply to the basilar system
 Chronic ischemia of the vertebral basilar arterial system due to
Atherosclerosis produces vertigo

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EARS, NOSE & THROAT (ENT)
Topic: Diseases of the Inner Ear
Lecturer: Dr. Caluag, Jim

Benign Paroxysmal Vertigo (BPPV) Other Management of Meniere’s Disease:


 Condition usually seen in adults when the patient’s head is placed in a  If the patient has INCAPACITATING VERTIGO in spite of extended
certain provocative position medical treatment, LABYRINTHECTOMY is done in which the inner ear
 A patient with this problem has nystagmus towards the affected ear. contents are surgically removed and surgical drainage of the
 Most evidence to date locates the causative lesion to involve the utricle. Endolymphatic sac to reduce swelling
 May follow trauma or may appear spontaneously  Another is IV administration of high doses of streptomycin which will
 Hearing and Caloric Testing are Normal primarily destroy the vestibular labyrinth, although hearing function
should be well monitored
 Management:
o Reassurance that the condition is not serious
o Antiemetics and sedatives can help

Vestibular Neuronitis
 Also known as epidemic labyrinthitis because it sometimes seems to
occur in epidemic form
 Patient presents with intermittent vertigo.
 Examination shows normal hearing but with a reduced caloric reaction
 Thought to be due to a virus that attacks the ganglion of the vestibular
nerve but attacks the whole vestibular labyrinth
 Thought to be due to the activation of Human Herpes simplex virus
 Medications are supportive but not specific.
 A self-limiting

Vertigo caused by Tumors


 Hearing loss and vertigo can be caused by tumors affecting the middle
or inner ear.
 Tumors of the temporal bone may be classified as primary or
secondary.
Primary Tumors:
 Glomus jugulare tumor of the middle ear
 Chemodectomas
 Squamous cell carcinoma of the ear canal and the middle ear
 Acoustic neuromas

 Management:
o Treatment after an early diagnosis is preferably surgical.
o Radiotherapy is a useful pre and post-operative adjunct
therapy.

MENIERE’S DISEASE (ENDOLYMPHATIC HYDROPS)


 There is an excessive amount of endolymph being produced which
results to an increase in the volume of the endolymph resulting to
swelling of the membranous labyrinth that will eventually cause
dizziness and deafness (sensorineural hearing loss).
 The cause is probably related to a disturbance in the osmotic pressure
gradient at the blood-endolymph barrier.
 Triad Symptoms of Meniere’s Disease:
o Tinnitus (Subjective)
o Deafness (SNHL)
o Vertigo (Episodic)

 Management:
o Diuretics are given to reduce the swelling of the membranous
labyrinth.
o It has the same principle of management with glaucoma.
o Sedatives
o Central vasodilators
o Antiemetics

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